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Multiple endocrine neoplasia (MEN) I

Wermer syndrome; MEN I

Multiple endocrine neoplasia (MEN) type I is disease passed down through families in which one or more of the endocrine glands are overactive or form a tumor. Endocrine glands most commonly involved include:

Pancreas

Parathyroid

Pituitary

Causes

MEN I is caused by a defect in a gene that carries the code for a protein called menin. The condition causes tumors of various glands to appear in the same person, but not necessarily at the same time.

The disorder may occur at any age, and it affects men and women equally. A family history of this disorder raises your risk.

Symptoms

Symptoms vary from person to person, and depend on which gland is involved. They may include:

Treatment

Surgery to remove the diseased gland is the treatment of choice. A medication called bromocriptine may be used instead of surgery for pituitary tumors that release the hormone prolactin.

The parathyroid glands, which control calcium production, can be removed. However, because it is difficult for the body to regulate calcium levels without these glands, a total parathyroid removal is usually not done initially.

Medicine is available todecrease the excess stomach acid production caused by some tumors, and to reduce the risk of ulcers.

Hormone replacement therapy is given when entire glands are removed or do not produce enough hormones.

Outlook (Prognosis)

Pituitary and parathyroid tumors are usually noncancerous (benign), but some pancreatic tumors may become cancerous (malignant) and spread to the liver. These can lower life expectancy.

Benign

"Benign" refers to a condition, tumor, or growth that isnot cancerous. This means that it does not spread to other parts of the body. It does not ...

Endocrine glands - illustration

Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

Endocrine glands

illustration

Endocrine glands - illustration

Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).